Causes and clinical manifestations of primary adrenal insufficiency in children
- Patricia A Donohoue, MD
Patricia A Donohoue, MD
- Professor of Pediatrics
- Medical College of Wisconsin
Adrenal insufficiency is defined by the impaired synthesis and release of adrenocortical hormones. It is classified based upon the mechanism:
●Primary adrenal insufficiency results from disease intrinsic to the adrenal cortex (table 1). These patients may present with glucocorticoid deficiency, with or without deficiencies of mineralocorticoids and adrenal androgens.
●Central adrenal insufficiency is caused by impaired production of adrenocorticotropic hormone (ACTH). It can be caused by either pituitary disease that impairs release of ACTH, or by interference with corticotropin releasing hormone (CRH) production from the hypothalamus. These patients have only glucocorticoid deficiency and not mineralocorticoid deficiency. In addition, they may have other pituitary hormone deficiencies, depending on the underlying central nervous system disease.
The causes and clinical manifestations of primary adrenal insufficiency will be discussed in this topic review. The central (secondary and tertiary) causes of adrenal insufficiency are discussed elsewhere in the program (see "Causes and clinical manifestations of central adrenal insufficiency in children"). The diagnosis and treatment of adrenal insufficiency is discussed separately. (See "Diagnosis of adrenal insufficiency in children" and "Treatment of adrenal insufficiency in children".)
The signs and symptoms of primary adrenal insufficiency vary depending on what class(es) of hormone are deficient and the severity of the defect(s). The adrenal hormones are divided into glucocorticoids (eg, cortisol), mineralocorticoids (eg, aldosterone), and adrenal androgens (eg, dehydroepiandrosterone [DHEA]).To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
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- CLINICAL OVERVIEW
- Glucocorticoid deficiency
- Mineralocorticoid deficiency
- Adrenal androgen deficiency
- Adrenal crisis
- Differential diagnosis
- DISORDERS OF STEROIDOGENESIS
- Congenital adrenal hyperplasia
- Defects in aldosterone production
- Defects in cholesterol biochemistry
- ADRENAL DAMAGE OR DYSFUNCTION
- Adrenal hemorrhage
- Autoimmune disease
- Mitochondrial disorders
- Critical illness-related corticosteroid insufficiency (CIRCI)
- Transient adrenal insufficiency in premature infants
- PEROXISOMAL DISORDERS
- Other causes
- ABNORMAL ADRENAL DEVELOPMENT
- Adrenal hypoplasia congenita
- Other causes
- INHERITED ADRENAL UNRESPONSIVENESS TO ACTH
- Familial glucocorticoid deficiency (FGD)
- Triple A syndrome
- END-ORGAN UNRESPONSIVENESS
- SOCIETY GUIDELINE LINKS
- INFORMATION FOR PATIENTS